Position statement on fetal myelomeningocele repair.

Following the promising multicenter randomized trial results of in utero fetal myelomeningocele repair; we anticipate that an increasing number of tertiary care centers may want to offer this therapy. It is essential to establish minimum criteria for centers providing open fetal myelomeningocele repair to ensure optimal maternal and fetal/pediatric outcomes, as well as patient safety both short- and long-term; and to advance our knowledge of the role and benefit of fetal surgery in the management of fetal myelomeningocele. The fetal myelomeningocele Maternal-Fetal Management Task Force was initially convened by the Eunice Kennedy Shriver National Institute of Child Health and Human Development to discuss the implementation of maternal fetal surgery for myelomeningocele. The decision was made to develop the optimal practice criteria presented in this document for the purpose of medical and surgical leadership. These criteria are not intended to be used for legal or regulatory purposes.

Awake craniotomy for supratentorial tumors or epileptogenic lesions in pediatric patients: a 16-year retrospective cohort study.

OBJECTIVE: Awake craniotomy with intraoperative mapping is the widely accepted procedure for adult patients undergoing supratentorial tumor or epileptogenic focus resection near eloquent cortex. In children, awake craniotomies are notably less common due to concerns for compliance and emotional or psychological repercussions. Despite this, successfully tolerated awake craniotomies have been reported in patients as young as 8 years of age, with success rates comparable to those of adults. The authors sought to describe their experience with pediatric awake craniotomies, including insight regarding feasibility and outcomes. METHODS: A retrospective review was completed for all pediatric (age < 18 years) patients at Children's Wisconsin for whom an awake craniotomy was attempted from January 2004 until March 2020. Institutional review board approval was granted. RESULTS: Candidate patients had intact verbal ability, cognitive profile, and no considerable anxiety concerns during neuropsychology assessment. Nine patients presented with seizure. Five patients were diagnosed with tumor and secondary epilepsy, 3 with tumor only, and 3 with epilepsy only. All patients who underwent preoperative functional MRI successfully completed and tolerated testing paradigms. A total of 12 awake craniotomies were attempted in 11 patients, with 1 procedure aborted due to intraoperative bleeding. One patient had a repeat procedure. The mean patient age was 15.5 years (range 11.5-17.9 years). All patients returned to or maintained baseline motor and speech functions by the latest follow-up (range 14-130 months). Temporary deficits included transient speech errors, mild decline in visuospatial reasoning, leg numbness, and expected hemiparesis. Of the 8 patients with a diagnosis of epilepsy prior to surgery, 7 patients achieved Engel class I designation at the 1-year follow-up, 6 of whom remained in class I at the latest follow-up. CONCLUSIONS: This study analyzes one of the largest cohorts of pediatric patients who underwent awake craniotomy for maximal safe resection of tumor or epileptogenic lesions. For candidate patients, awake craniotomy is safe, feasible, and effective in carefully selected children.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Current state and future direction of quality improvement in pediatric neurosurgery: a survey of pediatric neurosurgeons.

OBJECTIVE: Quality improvement (QI) is a methodology used to implement sustainable, meaningful change to improve patient outcomes. Given the complex pathologies observed in pediatric neurosurgery, QI projects could potentially improve patient care. Overall, there is a need to characterize the degree of QI opportunities, training, and initiatives within the field of pediatric neurosurgery. Herein the authors aimed to define the current QI landscape in pediatric neurosurgery. METHODS: A cross-sectional survey was sent to all members of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery via email. The responses were anonymized. Questions addressed several relatable QI topics including 1) training and participation in QI; 2) QI infrastructure; 3) QI program incentives; and 4) general opinions on the National Surgical Quality Improvement Program (NSQIP) database, various QI topics, and QI productivity. RESULTS: Responses were received from 129 participants (20% response rate). Most respondents practiced in an academic setting (59.8%) and at a free-standing pediatric hospital (65.4%). Participation in QI projects was high (81.7%), but only 23.8% of respondents had formal QI training. Only 36.5% of respondents had institutional requirements for QI work; the majority of those were only required to participate as a project team member. Nearly half of the respondents did not receive incentives or institutional support for QI. The majority agreed ("strongly" and "somewhat") that a QI course would be beneficial (75.5%), that QI projects should be considered for publication in neurosurgery journals (88.1%), and that there is a need for national quality metrics (81.4%). Over 88% have an interest in seeing QI project presentations at the annual Pediatric Joint Section meeting. Only 26.3% believed that the NSQIP was a useful QI guide. Respondents suggested further study of the following QI topics: overall rates of infection and their prevention, hydrocephalus, standardized treatment algorithms for common disorders, team communication, pediatric neurosurgery-specific database, access to care, and interprofessional education. CONCLUSIONS: Areas of opportunity include specialty-specific QI education, tactics for obtaining support to build the QI infrastructure, increased visibility of QI work within pediatric neurosurgery, and a review of available registries to provide readily available data relevant to this specialty.

Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

OBJECTIVE: The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS: The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS: A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS: Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Split laminotomy versus conventional laminotomy: postoperative outcomes in pediatric patients.

OBJECTIVE Split laminotomy is a technique for accessing the spinal canal from the posterior midline that minimizes muscle dissection and bone removal. Benefits of this approach in minimizing postoperative pain and muscle atrophy in the adult population have been reported, but pediatric data are limited. Herein, the authors evaluate the benefits of the split laminotomy technique in pediatric patients. METHODS Data obtained in patients who underwent posterior spine surgery at Children's Hospital of Wisconsin for an intradural midline pathology between April 2008 and June 2015 were reviewed retrospectively. Each patient was assigned to one of two groups, the split-laminotomy or conventional-laminotomy group. The primary outcomes assessed were mean daily pain score, total opioid use over a period of 72 hours after surgery, and the degree of paraspinal muscle atrophy and fat infiltration found on short-term (1-4 months) and long-term (1-4 years) follow-up spine MRI studies. RESULTS A total of 117 patients underwent lumbar-level surgery (83 conventional laminotomy, 34 split laminotomy), and 8 patients underwent thoracic-level surgery (4 in each group). No significant difference in the mean daily pain scores between groups was found. The daily opioid use was significantly lower in the split-laminotomy group on postoperative day 0 (POD0) and POD1 but not on POD2 (p = 0.01, 0.01, and 0.10, respectively). The total opioid use over the 72-hour postoperative period was significantly lower in the split-laminotomy group (p = 0.0008). The fat/muscle ratio was significantly higher in both the short-term and long-term follow-up periods in the conventional-laminotomy group (p = 0.01 and 0.0002, respectively). The rate of change of paraspinal muscle fat infiltration was significantly lower in the split-laminotomy group than in the conventional-laminotomy group (p = 0.007). The incidence of complications was not significantly different between groups (p = 0.08). CONCLUSIONS This study was of the largest series reported thus far of pediatric patients who underwent split laminotomy and the only controlled study that has involved children. The authors' results reinforce the short-term benefit of split laminotomy in minimizing acute postoperative pain and long-term benefits of decreasing muscle atrophy and fatty degeneration, which are known to be associated with the development of chronic pain and spinal instability. Additional efforts for assessing long-term effects in the development of chronic pain, spinal instability, and spinal deformity are still necessary.

Noninvasive measurement of cerebrospinal fluid flow using an ultrasonic transit time flow sensor: a preliminary study.

OBJECT Mechanical failure-which is the primary cause of CSF shunt malfunction-is not readily diagnosed, and the specific reasons for mechanical failure are not easily discerned. Prior attempts to measure CSF flow noninvasively have lacked the ability to either quantitatively or qualitatively obtain data. To address these needs, this preliminary study evaluates an ultrasonic transit time flow sensor in pediatric and adult patients with external ventricular drains (EVDs). One goal was to confirm the stated accuracy of the sensor in a clinical setting. A second goal was to observe the sensor's capability to record real-time continuous CSF flow. The final goal was to observe recordings during instances of flow blockage or lack of flow in order to determine the sensor's ability to identify these changes. METHODS A total of 5 pediatric and 11 adult patients who had received EVDs for the treatment of hydrocephalus were studied in a hospital setting. The primary EVD was connected to a secondary study EVD that contained a fluid-filled pressure transducer and an in-line transit time flow sensor. Comparisons were made between the weight of the drainage bag and the flow measured via the sensor in order to confirm its accuracy. Data from the pressure transducer and the flow sensor were recorded continuously at 100 Hz for a period of 24 hours by a data acquisition system, while the hourly CSF flow into the drip chamber was recorded manually. Changes in the patient's neurological status and their time points were noted. RESULTS The flow sensor demonstrated a proven accuracy of ± 15% or ± 2 ml/hr. The flow sensor allowed real-time continuous flow waveform data recordings. Dynamic analysis of CSF flow waveforms allowed the calculation of the pressure-volume index. Lastly, the sensor was able to diagnose a blocked catheter and distinguish between the blockage and lack of flow. CONCLUSIONS The Transonic flow sensor accurately measures CSF output within ± 15% or ± 2 ml/hr, diagnoses the blockage or lack of flow, and records real-time continuous flow data in patients with EVDs. Calculations of a wide variety of diagnostic parameters can be made from the waveform recordings, including resistance and compliance of the ventricular catheters and the compliance of the brain. The sensor's clinical applications may be of particular importance to the noninvasive diagnosis of shunt malfunctions with the development of an implantable device.

An Optimized Voriconazole Dosing Strategy to Achieve Therapeutic Serum Concentrations in Children Younger than 2 Years Old.

STUDY OBJECTIVE: To describe our experience with voriconazole in three patients younger than 2 years using an optimized dosing strategy for voriconazole that incorporates intensive therapeutic drug monitoring (TDM). DESIGN: Case series. SETTING: Large pediatric hospital. PATIENTS: Three patients younger than 2 years who received voriconazole therapy and had serum trough concentrations measured between January 1, 2010, and October 31, 2015. MEASUREMENTS AND MAIN RESULTS: A clinical practice guideline developed at our institution was used to standardize initial dosing, appropriate use and timing of TDM, and dosage modifications based on TDM. TDM was used to guide dosing to achieve a target voriconazole serum trough concentration of 2-6 µg/ml. Voriconazole samples were assayed by using a high-performance liquid chromatography analytical method with solid-phase extraction. Initial dosages for the three patients were 9 mg/kg intravenously every 12 hours (one patient) and 9 mg/kg enterally twice/day (two patients). Multiple dose escalations and a more frequent dosing interval were required to achieve trough concentrations within the target range. The final dosages were 12 mg/kg intravenously every 8 hours, 17.7 mg/kg enterally 3 times/day, and 8.5 mg/kg enterally 3 times/day, respectively. In addition to voriconazole trough concentrations, TDM included evaluations for drug toxicities. Visual, neurologic, or hepatic adverse effects were not encountered in the three patients. CONCLUSION: Our data support higher initial doses and perhaps a 3 times/day dosing schedule to achieve voriconazole serum concentrations in the target range for children younger than 2 years. Implementation of a clinical practice guideline with the participation of pharmacists specializing in pharmacokinetics allows for effective use of voriconazole in young children.

Mycoplasma hominis and Ureaplasma species brain abscess in a neonate.

We report an infant with a mixed Mycoplasma and Ureaplasma brain abscess who was successfully treated with intravenous doxycycline and erythromycin. Therapeutic concentrations of doxycycline were demonstrated in cerebrospinal fluid. This report is evidence of the potential for Mycoplasma and Ureaplasma to produce focal central nervous system infection, as well as meningitis, in neonates and that use of doxycycline can be efficacious in the therapy of such infections.

The circle of sagittal synostosis surgery.

Sagittal synostosis, the premature closure of the sagittal suture, accounts for more than 50% of all nonsyndromic single-suture synostoses. Although no detrimental neurologic effects can be directly attributed to the synostosis, a number of patients will have relatively increased intracranial pressure. Surgical correction for sagittal synostosis has evolved from simple removal of bone strips to extensive cranial remodeling, all in a attempt to achieve a normal head shape. The lack of outcome measures has limited the surgeon's ability to choose one surgical procedure over another. The use of a cranial helmet for passive molding of the head after surgery is effective when used with limited endoscopic surgery. We present the results of using an extended strip craniectomy combined with long-duration molding helmet therapy and compare it with other reported methods. The results suggest that passive molding with the helmet may have a greater effect on cranial shape than surgery.

Neural tube defects.

Defects of development of the neural tube can result in a number of seemingly different malformations. Understanding the abnormal embryology helps one understand the malformations and their surgical treatments. The clinical presentations and the follow-up of these patients require attention to various end organs besides the nervous system. For most of these conditions, long-term follow-up is necessary regardless of initial treatment. A decline in function is not a part of the natural history of these malformations and requires prompt evaluation and treatment.

Minicraniotomy versus bur holes for evacuation of chronic subdural collections in infants-a preliminary single-institution experience.

OBJECT: Various surgical interventions have been described to evacuate chronic subdural collections (CSCs) of infancy. These include transfontanel percutaneous aspiration, subdural drains, placement of bur hole(s) with or without a subdural drain, and shunting. Shunt placement typically provides good long-term success (resolution of the subdural fluid), but comes with well-known early and late complications. Recently, the authors have used a mini-osteoplastic craniotomy technique with the goal of definitively treating these children with a single surgery while avoiding the many issues associated with a shunt. They describe their procedure and compare it with the traditional bur hole technique. METHODS: In this single-institution retrospective study, the authors evaluated 26 cases involving patients who underwent treatment for CSC. Preoperative, intraoperative, and postoperative data were reviewed, including radiographic findings (density of the subdural fluid and ventricular and subarachnoid space size), neurological examination findings, and intraoperative fluid description. The primary outcome was treatment failure, defined as the patient requiring any subsequent surgical intervention after the index procedure (minicraniotomy or bur hole placement). RESULTS: Fifteen patients (10 male and 5 female; median age 5.1 months) collectively underwent 27 minicraniotomy procedures (each procedure representing a hemisphere that was treated). In the bur hole group, there were 11 patients (6 male and 5 female; median age 4.6 months) with 18 hemispheres treated. Both groups had subdural drains placed. The average follow-up for each treatment group was just over 7 months. Treatment failure occurred in 2 patients (13%) in the minicraniotomy group compared with 5 patients (45%) in the bur hole group (p = 0.09). Furthermore, the 2 patients who had treatment failure in the minicraniotomy group required 1 subsequent surgery each, whereas the 5 in the bur hole group needed a total of 9 subsequent surgeries. Eventually, 80% of the patients in the minicraniotomy group and 70% of those in the bur hole group had resolution of the subdural collections on the last imaging study. CONCLUSIONS: The minicraniotomy technique may be a superior technique for the treatment of CSCs in infants compared with bur hole evacuation. The minicraniotomy provides greater visualization of the subdural space and allows more aggressive evacuation of the fluid, better irrigation of the space, the ability to fenestrate any accessible membranes safely, and continued egress of fluid into the subgaleal space. Although this preliminary report has obvious limitations, evaluation of this technique may be worthy of a prospective, multiinstitutional collaborative effort.

Spinal dural closure with nonpenetrating titanium clips in pediatric neurosurgery.

OBJECT: The authors report a retrospective review of their experience using nonpenetrating titanium anastomotic clips for dural closure in 27 pediatric cases (26 patients) of spinal surgery for a variety of diagnoses. The goal of this review was to define the utility of these clips in pediatric neurosurgical spinal procedures, identify complications of their use, and assess the effects on postoperative imaging because of their use. METHODS: Institutional review board approval was obtained for a retrospective chart review of all patients in whom titanium dural clips had been utilized. Patients were identified over a 2-year period using hospital and clinic records, and data were collected on the patient demographics, surgical diagnosis and procedure, durotomy location and length, and adjunctive closure methods. Postoperative complications were assessed. When available, postoperative imaging data were reviewed. RESULTS: Twenty-six patients underwent 27 operations over a 20-month period. They ranged in age from 2.5 months to 18.5 years, with a median age of 3.2 years and an average age of 5.8 years. The operative diagnosis was some form of spinal dysraphism in 19 patients, with a syrinx or dural tear in 2 patients each, and an arachnoid cyst in 3 cases; 1 patient had a tumor resected. Operative levels included lumbosacral (19), thoracic (7), and cervical (1). Dural exposure was limited to 1 laminar level in 16 cases, 2 levels in 8, and 3 levels in 1; 2 cases involved focal dural tears. A combination of additional hemostatic and tissue sealant materials was applied over the clips in 16 cases. One patient required reoperation 13 months after clip placement. Prior clip use did not make subsequent exposure and opening more complicated. No significant complications were identified in the follow-up period ranging from 1 to 24 months. There were no documented CSF leaks. The clips are not easily seen on plain radiographs and did not cause artifacts or distortion on either CT or MR imaging. CONCLUSIONS: Nonpenetrating titanium anastomotic clips afford an effective means of closure while limiting the exposure needed, and thus allowing more minimally invasive approaches. In tight spaces, dural closure is accomplished more easily and faster with the clips as compared with conventional suturing. No significant complications were seen from clip use, and the clips did not interfere with postoperative imaging.

Modified osteoplastic orbitozygomatic craniotomy in the pediatric population.

OBJECT: Anterior and anterolateral skull base approaches offer the advantages of improved visualization and minimal brain retraction for lesions involving the orbital apex, parasellar regions, and anterior and middle fossa floors. These approaches are seldom used in the pediatric population due to the perceived increase in morbidity and surgical complexity. We report the application of the previously described modified osteoplastic orbitozygomatic (OZ) craniotomy to pediatric neurosurgical cases. This approach offers a number of advantages and is technically straightforward. MATERIALS AND METHODS: The results from six pediatric cases are reported. Age ranged from 26 months to 15 years, with a follow-up period of 5 to 22 months. Pathology included craniopharyngioma (three), frontal epidural abscess-subdural empyema with intraorbital extension (one), hypothalamic hamartoma (one), and optic pathway glioma (one). No complications related to the surgical approach were noted. In all cases, good postoperative cosmesis was achieved with excellent realignment of the orbital rim. Temporalis muscle bulk was preserved and symmetric in all cases. CONCLUSION: The modified osteoplastic OZ craniotomy can be safely and effectively applied to the pediatric population. Advantages include: (1) ease of use; (2) superior exposure and therefore less brain retraction; (3) an easily replaced one-piece bone flap which obviates the need for plating-suturing at the orbital rim; (4) a vascularized bone flap less susceptible to infection; and (5) maintenance of normal temporalis muscle anatomy for improved cosmesis and function.

Clinical significance of terminal syringomyelia in association with pediatric tethered cord syndrome.

An association between terminal syringomyelia (TS) and tethered cord syndrome (TCS) has been recognized. The clinical significance of TS and the need for concurrent treatment is not known. Thirty-four patients with TCS undergoing surgery for tethered cord release were evaluated for the incidence of TS. The clinical and radiological response to untethering with and without syrinx drainage was assessed. The group incidence of TS was 29.4%. There were no significant differences in the sex, age, underlying pathology or preoperative symptoms between the TCS group (TCS, n = 24) and the TCS group with TS (TCS + TS, n = 10). In the TCS group, 37.5% of the patients were asymptomatic preoperatively, and in the TCS + TS group 50% were asymptomatic preoperatively. After surgery, none of these asymptomatic patients developed symptoms. All of the symptomatic TCS + TS patients improved clinically, 12 of 15 symptomatic TCS patients improved, 2 patients were unchanged and 1 had worsening scoliosis. Syrinx did not develop in patients lacking it preoperatively. In patients where postoperative imaging was available, preoperative syringes improved. TS is clearly associated with TCS. Tethered cord release alone may be sufficient to improve preoperative symptoms and TS may be an associated phenomenon that does not mandate separate treatment.

Outcome analysis of initial neonatal shunts: does the valve make a difference?

OBJECTIVE: Ventriculoperitoneal shunts have one of the highest complication rates of all neurosurgical procedures. The purpose of this study was to identify factors associated with malfunction of shunts placed in infants with neonatal hydrocephalus, with the goal of maximizing long-term shunt survival. METHODS: We performed a retrospective chart review of 200 consecutive patients less than 1 year old who underwent primary intracranial shunt placement for hydrocephalus by one of two experienced pediatric neurosurgeons at a single institution. A multivariate analysis was conducted to identify variables that were statistically independent predictors of a shunt malfunction or problem. RESULTS: Adequate data were available for 158 patients, with a mean follow-up of 39.8 months (range 6-99 months). Variables tested for independent prediction of shunt revision included the etiology of the hydrocephalus, gestation period, age at shunt placement, surgeon, ventricular catheter entry site and valve opening pressure. Frontal versus occipital catheter entry site was not associated with a different revision rate. The only significant controllable factor associated with shunt malfunction was the valve opening pressure. The revision rate per year of follow-up was 4 times higher for patients with no valve or a low-pressure valve than for patients with a medium- or high-pressure valve. CONCLUSIONS: This retrospective review demonstrated that the valve opening pressure is an important component of the shunt complication rate. A prospective multicenter randomized trial is warranted to further evaluate the conclusions of this study.